Hair abnormalities menkes syndrome

Menkes disease patients are unable to absorb copper and hence suffer from the effect of copper deficiency. The disease has a very early age of onset and affected children suffer from neurodegeneration and die before the age of three years. All patients gain very little weight following birth. Several gross abnormahties are noted with hair. They are coarse and brittle and either twisted or fractured at various intervals. This abnormality of hair in Menkes disease led to the disorder to be referred to as kinky hair syndrome. 

Menkes syndrome is linked to a copper deficiency resulting in abnormal keratinization [167]. In this genetic disorder, the kinky hair symptomatic of this disease results from an unusually high mercaptan level of cysteine, wherein only about 50% of the cysteine is oxidized to disulfide bonds during keratinization. 

Monilethrix is a genetic anomaly in which hair fibers contain periodic constrictions along the fiber axis. Monilethrix hairs tend to fracture at these constrictions [63] and therefore must exhibit abnormal stretching behavior. Trichorrhexis nodosa is another abnormal condition, where hair fibers contain nodes at irregular intervals along the fiber axis. These nodes actually contain tiny fractures and tend to form broom like breaks [63] under stress. Therefore, nodosa hair fibers should also exhibit abnormal stretching behavior. Other hair shaft anomalies such as trichothiodystrophy and Menkes syndrome should also display abnormal stretching behavior consistent with the abnormal hair shaft condition associated with these diseases. 

With the exception of anemia, all the pathological abnormalities associated with copper deficiency in experimental animals have been observed in patients with Menkes syndrome (Banks, 1975). In fact, the similarity between wool from copper-deficient sheep and hair from Menkes patients prompted Banks et al. (1973a) to suggest that the term steely hair be used to describe the hair on these affected infants. 

Copper Anaemia Menkes (kinky hair) syndrome Cardiac abnormalities/heart disease Wilson s disease Hepatic injury and jaundice Headache, vomiting Haemolytic shock... 

Menkes disease is a genetic disease involving mental retardation and death before the age of 3 years. The disease is also called Menkes steely hair syndrome. This term came from the abnormal, steely or kinky hair that results. The hair is tangled, grayish, and easily broken. 

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